Management of Solid Pseudopapillary Neoplasm of the Pancreas

Solid pseudopapillary neoplasm (SPN) of the pancreas is a rare, low-grade malignant tumor that predominantly affects young women in the second to fourth decades of life. It accounts for 1–2% of all pancreatic neoplasms and has very different clinical, radiological, and pathological features, which differentiate it from other cystic pancreatic lesions. SPN is mainly composed of a large, well-encapsulated mass with mixed solid and cystic components and is frequently an incidental finding or presents with symptoms like abdominal pain, indigestion, and a palpable mass. Radiologically, the tumor has an irregular enhancement pattern with areas of hemorrhage and necrosis, while histopathologically, it shows pseudopapillary structures consisting of uniform epithelial cells with nuclear grooves and cytoplasmic vacuolation. The immunohistochemical staining for β-catenin (nuclear and cytoplasmic) and vimentin is positive in majority of cells and helps in confirming the diagnosis. Complete surgical removal of the tumor is the standard of care and is associated with an almost complete cure, the 5-year survival rates exceeding 95%. This chapter on SPN elucidates its characteristics, diagnostic work-up, imaging features, along with histopathological profile and management strategies.